Mutation in leads to early motor dysfunction and widespread aberrant axon terminals in a beta-propeller protein associated neurodegeneration (BPAN) patient-inspired mouse model.

Beta-propeller Protein Associated Neurodegeneration (BPAN) is a devastating neurodevelopmental and neurodegenerative disease linked to variants in . Currently, there is no cure or disease altering treatment for this disease. This is, in part, due to a lack of insight into early phenotypes of BPAN progression and 's role in establishing and maintaining neurological function.

Primary Intracranial Sarcoma, DICER1-Mutant, With Prominent Chondroid Differentiation: Case Report and Summary of Reported Patients in Literature.

Primary intracranial sarcoma, -mutant, included as a new diagnostic entity in the 2021 WHO Classification of Central Nervous System Tumors, is a rare, but aggressive neoplasm generally identified in the supratentorial forebrain. The prognostic implications of these uncommon tumors and optimal treatment strategy remain unclear. A 19-year-old woman was found unresponsive after reporting a severe headache.

Tracing the transmission of carbapenem-resistant Enterobacterales at the patient: ward environmental nexus.

Introduction: Colonisation and infection with Carbapenem-resistant Enterobacterales (CRE) in healthcare settings poses significant risks, especially for vulnerable patients. Genomic analysis can be used to trace transmission routes, supporting antimicrobial stewardship and informing infection control strategies. Here we used genomic analysis to track the movement and transmission of CREs within clinical and environmental samples.

In-vitro and in-use efficacy of a rapid, targeted UVC decontamination system.

Background: Ultraviolet C (UVC) decontamination systems are used in healthcare to supplement manual cleaning. Systems typically target whole rooms with fixed, vertical bulbs.

Aim: To evaluate the in-vitro and in-use efficacy of the novel device with emitters on articulating arms designed for rapid, targeted decontamination in a hospital room.

Evaluation of novel disinfection methods for the remediation of heavily contaminated thermostatic mixing valves and water systems with Pseudomonas aeruginosa biofilm: considerations for new and existing healthcare water systems.

Background: Pseudomonas aeruginosa is a leading cause of nosocomial Gram-negative bacteraemia. Water systems are a well-documented source of P. aeruginosa and established biofilms are difficult to remove.

The need for in-house efficacy testing and assessment of the antimicrobial properties of silver nanoparticle (Ag)-impregnated showerheads against Pseudomonas aeruginosa intended for healthcare waters in high-risk patient settings.

Introduction: Pseudomonas aeruginosa is a Gram-negative water-borne opportunistic pathogen. Reservoirs of bacteria can infect vulnerable populations. Mitigation of this risk in health care includes regular microbiology monitoring and chemical/thermal disinfection of outlets.

EUS-Guided Cholecystoduodenostomy for a Poor Surgical Candidate With Chronic Octreotide-Associated Gallstones From Metastatic Neuroendocrine Tumor.

Chronic octreotide use has been associated with gallstone formation. Historically, cholecystectomy has been the defining treatment for those who have gallstone-related disease. For those who are poor surgical candidates, percutaneous and endoscopic approaches have been used.

A MoClo-Compatible Toolbox of ECF Sigma Factor-Based Regulatory Switches for Proteobacterial Chassis.

The construction of complex synthetic gene circuits with predetermined and reliable output depends on orthogonal regulatory parts that do not inadvertently interfere with the host machinery or with other circuit components. Previously, extracytoplasmic function sigma factors (ECFs), a diverse group of alternative sigma factors with distinct promoter specificities, were shown to have great potential as context-independent regulators, but so far, they have only been used in a few model species. Here, we show that the alphaproteobacterium , which has been proposed as a plant-associated bacterial chassis for synthetic biology, has a similar phylogenetic ECF acceptance range as the gammaproteobacterium .

Concordance between IDEXX Legiolert® (liquid culture assay) and plate culture (ISO 11731:2017) for the detection and quantification of Legionella pneumophila in water samples.

Background: Legionella pneumophila is a water-borne bacterium that can cause Legionnaires' disease. Legiolert® (IDEXX, USA) is a low-labour liquid culture assay for the detection and enumeration of L. pneumophila (SG1-15) from water.

Enhanced monitoring of healthcare shower water in augmented and non-augmented care wards showing persistence of despite remediation work.

in healthcare shower waters presents a high risk of infection to immune-suppressed patients; identifying the colonization-status of water outlets is essential in preventing acquisition. Testing frequencies may be insufficient to capture presence/absence of contamination in healthcare waters between sampling and remediation activities. Standardization of outlets may facilitate the management and control of .

Failure of a hollow-fibre shower filter device to prevent exposure of patients to Pseudomonas aeruginosa.

Background: Pseudomonas aeruginosa in hospital water is a risk for invasive infection. Point-of-use (POU) filters are used to reduce patient exposure to the organism, and hollow-fibre filters are becoming more popular. However, retrograde colonization of the filter mechanism may contaminate the effluent.

Metastatic Colon Cancer in the Pituitary: A Case Report with Review of Literature.

We report a rare case of metastatic colonic adenocarcinoma to the pituitary gland in a 58-year-old who presented with visual decline and panhypopituitarism. He underwent urgent transsphenoidal endoscopic surgery with significant improvement of his vision, followed by adjuvant fractionated radiotherapy to the resection cavity. He made a satisfactory recovery, but regrettably died from COVID-19 9 weeks after completion of radiotherapy.

Cerebrospinal fluid shunting protocol for idiopathic intracranial hypertension for an improved revision rate.

Objective: Cerebrospinal fluid (CSF) shunting in idiopathic intracranial hypertension (IIH) is associated with high complication rates, primarily because of the technical challenges that are related to small ventricles and a large body habitus. In this study, the authors report the benefits of a standardized protocol for CSF shunting in patients with IIH as relates to shunt revisions.

Methods: This was a retrospective study of consecutive patients with IIH who had undergone primary insertion of a CSF shunt between January 2014 and December 2020 at the authors' hospital.

Thermal disinfection at suboptimal temperature of Pseudomonas aeruginosa biofilm on copper pipe and shower hose materials.

Background: Hospital-acquired infections caused by Pseudomonas aeruginosa have been linked to contaminated shower systems in health care. Thermal disinfection, whereby colonized outlets are flushed with existing hot water supplies, is a commonly used method to disinfect contaminated systems. Temperatures of 60°C are recommended for inactivation of P.

A 24-month cost and outcome analysis comparing traditional fronto-orbital advancment and remodeling with endoscopic strip craniectomy and molding helmet in the management of unicoronal craniosynostosis: A retrospective bi-institutional review.

Introduction: Endoscopic strip craniectomy with helmeting (ESCH) has been shown to be a safe and efficacious alternative to fronto-orbital remodeling (FOR) for selected children with craniosynostosis. In addition to clinical factors, there may be economic benefits from the use of ESCH instead of FOR.

Methods: A retrospective review of 23 patients with nonsyndromic unicoronal craniosynostosis (UCS) treated with FOR was carried out at Great Ormond Street Hospital (GOSH) for Children in London, UK.

Evaluation of droplet production by a new design of clinical handwash basin for the healthcare environment.

Splashing from handwash basins may be a source of bacteria in the healthcare environment. A novel splash-reducing basin was assessed for its ability to reduce droplet formation during simulated handwashing. The basin was compared to two conventional basins commonly used in healthcare.

EUS-guided drainage of pancreatic fluid collections using lumen apposing metal stents: An international, multicenter experience.

Introduction: Lumen apposing metal stents (LAMS) have been used increasingly for drainage of pancreatic fluid collections (PFC). We present an international, multicenter study evaluating the safety and efficacy of LAMS in PFCs.

Methods: Consecutive patients undergoing LAMS placement for PFC at 12 international centers were included (ClinicalTrials.

Cardiovascular Risks Associated with Gender and Aging.

The aging and elderly population are particularly susceptible to cardiovascular disease. Age is an independent risk factor for cardiovascular disease (CVD) in adults, but these risks are compounded by additional factors, including frailty, obesity, and diabetes. These factors are known to complicate and enhance cardiac risk factors that are associated with the onset of advanced age.

Uterine Lipoleiomyoma and Lipoma: A Rare Unique Case Report with Review of Literature.

Uterine lipoleiomyoma and pure lipoma are uncommon and extremely rare benign lipomatous tumors. Reported incidence is 0.03%-0.

Evaluation of Two Mobile Health Apps in the Context of Smoking Cessation: Qualitative Study of Cognitive Behavioral Therapy (CBT) Versus Non-CBT-Based Digital Solutions.

Background: Mobile health (mHealth) apps can offer users numerous benefits, representing a feasible and acceptable means of administering health interventions such as cognitive behavioral therapy (CBT). CBT is commonly used in the treatment of mental health conditions, where it has a strong evidence base, suggesting that it represents an effective method to elicit health behavior change. More importantly, CBT has proved to be effective in smoking cessation, in the context of smoking-related costs to the National Health Service (NHS) having been estimated to be as high as £2.

Cirrhotic Patients Have Worse Bowel Preparation at Screening Colonoscopy than Chronic Liver Disease Patients without Cirrhosis.

Background: Cirrhosis has been shown in small studies to be a predictor of suboptimal bowel preparation at screening colonoscopy. It has yet to be established whether patients with chronic liver disease in the absence of cirrhosis experience equally poor colon cleansing. Intestinal dysmotility related to cirrhosis might impair bowel preparation in this population more than those with chronic liver disease without cirrhosis.